ABSTRACT
Relapsing polychondritis is a rare multisystem autoimmune disorder of unknown etiology that was first described by Jaksch-Wartenhorst in 1923. 1 It is an episodic and progressive inflammatory disease of the cartilaginous structures, including the elastic cartilage of the ear and nose, the hyaline cartilage of peripheral joints, the fibrocartilage at axial sites, and cartilaginous structures of the tracheobronchial tree. Inflammation of other proteoglycan-rich structures such as the eyes, heart, blood vessels, inner ear, and kidneys may also occur. Since relapsing polychondritis was first described, more than 300 cases of the disease have been reported. The diagnosis is based on the clinical findings and may be confirmed histologically by biopsy of the affected ear. The disease is treated by corticosteroids and immunsuppressive agents. Here, we report on a woman suffering from refractory relapsing polychondritis whom we successfully treated by autologous stem cell transplantation (ASCT), resulting in a long-term remission which has lasted for 55 months now. We, therefore, conclude that ASCT may be of therapeutic benefit to patients with relapsing polychondritis who failed to respond to conventional therapy. Eligibility criteria for ASCT in relapsing polychondritis are discussed.
